Pemphigus foliaceus is a clinical form of pemphigus, which is characterized by the formation of flat unstrained bubbles, the reappearance of bubbles under the crusts in place of already resolved elements and pronounced delamination of the epidermis. The course of the disease is chronic, long-term, with a tendency to generalization and damage to extensive areas of the skin. Accurate diagnosis and differentiation of the disease from other forms of pemphigus is carried out during histological examination. With pemphigus foliaceus, therapy with immunosuppressive drugs (corticosteroid hormones, cytostatics), local treatment is carried out.
General information
The pemphigus foliaceus got its name due to the characteristic layered crusts covering the erosion formed in place of the bubbles. In the structure of dermatoses, the incidence of pemphigus foliaceus does not exceed 1.5%. Pemphigus foliaceus is the most common form of pemphigus in children. In adults, it predominates over vegetative pemphigus. Like other types of pemphigus, pemphigus foliaceus has an autoimmune mechanism of development with the formation of autoantibodies to epidermal cells. Without adequate therapy, patients may die from the addition of a secondary infection, cachexia.
Symptoms
Pemphigus foliaceus begins with the appearance of reddened skin areas with flattened flabby blisters located on them. In some cases, the onset of the disease proceeds according to the type of vulgar pemphigus, During dermatitis or resembles erythematous changes in eczema, toxicoderma, seborrheic dermatitis. Sometimes the blisters are not pronounced, and the exfoliation of the epidermis is defined as an area with an uneven surface.
The bubbles have a thin tire and burst quickly. Bright red erosions are formed with abundant discharge, which dries up with the formation of crusts. New portions of the separated form new layers of crusts. As a result, the elements characteristic of pemphigus foliaceus are formed, covered with layered crusts and resembling puff pastry.
The main distinguishing feature of the pemphigus foliaceus is the weakening of the connection between the granular and horny layers of the epidermis, which manifests itself even in apparently healthy areas of the skin. This causes a sharply positive symptom of Nikolsky — detachment of the epidermis for a considerable distance when pulling on the remnants of the tire of the burst bladder. For the same reason, even minor superficial damage to unchanged skin areas leads to delamination of the epidermis with the formation of blisters.
Erosions heal slowly. Often new blisters form under the crusts and this process can continue constantly, which is also a clinical feature of the pemphigus foliaceus. With the progression of the disease, individual adjacent bubbles tend to merge with the formation of extensive lesions.
The general condition of patients with pemphigus foliaceus at the beginning of the disease may be normal. As the area of the affected areas of the skin increases, it worsens. Even minor movements begin to cause pain to the patient, which is why the psyche suffers and sleep disorders occur. Temperature rises are noted, patients lose appetite and lose weight, cachexia develops. When the process spreads to the scalp, hair loss occurs. Nails fade, thicken and may fall out. The defeat of the oral mucosa is extremely rare.
Diagnostics
Often, the diagnosis of pemphigus foliaceus can be made on the basis of the clinical features of the disease described above. In smears-prints from the surface of erosions, acantholytic cells characteristic of all types of pemphigus are found, that is, cells with impaired intercellular connections. Histological examination reveals the presence of cracks or blisters between the horny and granular layer of the epidermis, the rupture of connections between cells (acantholysis). In old foci there is parakeratosis and hyperkeratosis, degeneration of cells of the granular layer of the skin.
Of the additional diagnostic methods, immunological studies are used to confirm the autoimmune mechanism of the development of the disease. However, the reactions of direct and indirect immunofluorescence (RIF) do not allow us to accurately distinguish pemphigus foliaceus from vulgar.
Pemphigus foliaceus is differentiated from other forms of pemphigus, as well as from During dermatitis, erythroderma, Lyell’s syndrome and other diseases.
Treatment and prognosis
Treatment is carried out under the supervision of a dermatologist mainly with glucocorticosteroids: dexamethasone, prednisone, methylprednisolone, triamcinolone, etc. In severe cases, therapy is supplemented with immunosuppressants (azathioprine, methotrexate). A positive effect was noted in the combination of corticosteroid drugs with synthetic antimalarial agents (hydroxychloroquine, chloroquine, etc.). Locally recommended baths with anti-inflammatory, disinfectants or astringent additives (for example, decoction of oak bark).
The prognosis is unfavorable. Against the background of treatment, the disease can occur for a long time. But in the end, the patient dies from cachexia, joined infections or complications of constant glucocorticoid therapy.